AB0838 PULMONARY AND CARDIOVASCULAR MANIFESTATIONS IN SYSTEMIC SCLEROSIS: RESULTS FROM A SSC PATIENTS COHORT AT A TERTIARY CENTRE OF NW GREECE
Authors: N. Gerolymatou, N. Koletsos, M. Karakosta, T. E. Memi, A. Drosos, P. Voulgari
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> Systemic Sclerosis (SSc) is a connective tissue disease affecting multiple systems, including the respiratory and cardiovascular systems. Among the various disease complications, interstitial lung disease (ILD), pulmonary arterial hypertension (PAH) and -lately recognized- myocardial fibrosis (MF), are those mainly related to reduced life expectancy. <h3>Objectives</h3> We aimed to evaluate the prevalence of SSc complications and to investigate possible associations with patients' clinical and serological characteristics. <h3>Methods</h3> Patients fulfilling the ACR/EULAR 2013 Scleroderma criteria, who were followed at our tertiary rheumatology centre between January 2018 and December 2022, were included in our cohort. Demographic, clinical, and serological characteristics, as well as imaging data (high-resolution chest CT, cardiac MRI) were used in our retrospective analysis. <h3>Results</h3> Ninety-one patients were included in our study, with 39.6% of them having diffuse cutaneous SSc. Most patients were female (81.3%), with a mean age at diagnosis 50.3 ±14.5 years and a mean disease duration 10.2 ±9.0 years. Leaving aside Raynaud's phenomenon and sclerodactyly, gastrointestinal involvement (71.4%), ulcers (46.2%) and arthritis (45.1%) were among the most prevalent clinical manifestations. Mean baseline FEV1 and FVC values were normal (83.4%±16.8 and 84.4%±18.5 respectively, while mean baseline DLCO was pathological (62.6±18.9). All patients underwent a high-resolution CT early at diagnosis and upon rheumatologist's/pulmonologist's judgement afterwards. Eighty-one patients (89.0%) were diagnosed with interstitial lung disease (76.0% had NSIP pattern, 24.0% UIP pattern). Patients with NSIP had better scores in lung function tests than those with UIP pattern (Mean difference in FEV1, FVC and DLCO were 13,3%, 17,7%, and 12% respectively, p&lt;0.05 for all comparisons). Positivity for anti-topoisomerase antibody (Scl-70) presented an OR=24.9 (p=0.001) for ILD, while ACA and anti-CENP-B were negatively associated (OR=0.114, p=0.003 and OR=0.164, p=0.024, respectively). Pulmonary hypertension based on echocardiogram findings was reported in 18/91 patients (19,8%), however PAH based on right heart catheterization (RHC) was confirmed in 11 of them (12,1% of total patients). Anti-Ro52 positivity was associated with PH (OR=4.45, p=0,005) and PH-ILD coexistence (OR=5.18, p=0,002), however no association was found for ILD presence in this cohort. There was also a trend for PAH-RHC, but it did not reach statistical significance. Cardiac MRI was performed in 24 patients, and non-ischemic myocardial fibrosis (compatible with SSc involvement) was identified in 18/24. The mean disease duration in MF group was longer (8.7 ±7.3 vs 3.3 ±4.3, p=0.040). Gastrointestinal involvement seems to confer an increased risk for this complication (OR=10.0, p=0.038). <h3>Conclusion</h3> The estimated ILD prevalence in our study is significantly higher than the one reported in the literature. As a referral centre, the patients reaching to our department probably have worse organ involvement than the average patient of primacy care setting. In addition, universal screening with a baseline HRCT and close monitoring lead to an earlier identification of this population. Anti-Ro52 has been recently reported as an independent risk factor for PAH. Our results could not verify this observation, probably due to the relatively small number of PAH patients in our study. Cardiac MRI availability and radiologist's experience are essential for MF diagnosis. It is our belief that larger studies are necessary to evaluate this population. <h3>References</h3> [1]Khanna SA, Nance JW, Suliman SA. Detection and Monitoring of Interstitial Lung Disease in Patients with Systemic Sclerosis. Curr Rheumatol Rep. 2022 May;24(5):166-173. [2]AYS Lee, KA Patterson et al (2021) Anti-Ro52/TRIM21 is independently associated with pulmonary arterial hypertension and mortality in a cohort of systemic sclerosis patients, Scand J Rheumatol, 50:6, 469-474. <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> None Declared.